Hameed Nazrin, Keshri Amit, Manogaran Ravi Sankar, Srivastava Arun K, Chidambaram Kalyana S, Aqib Mohd, Das Nidhin, Sinha Mohit
1Neurotology Unit, Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow; and.
2Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
J Neurosurg Pediatr. 2025 Jan 3;35(4):407-416. doi: 10.3171/2024.9.PEDS24362. Print 2025 Apr 1.
The objective of this study was to discuss the characteristics of intracranial extension in patients with juvenile nasopharyngeal angiofibroma (JNA) and propose and an algorithm for its management.
A retrospective chart review of all patients with JNA who underwent operations between January 2013 and January 2023 was done, and those cases with intracranial extension categorized as stage IIIb, IVa, and IVb according to the Andrews modification of the Fisch staging classification were included in the study. Data were collected about age at presentation, symptoms, radiological findings, routes of intracranial extension, therapeutic management, and follow-up.
Of 142 patients who underwent surgery for JNA, there were 40 (28.2%) cases with intracranial involvement. All patients were male with ages ranging from 10 to 26 years, with a mean age of 17 years at presentation. According to Andrews-Fisch classification, 28 patients presented with stage IIIb, 10 patients with stage IVa, and 2 patients with stage IVb. Parasellar involvement via the superior orbital fissure was the most frequent route of intracranial spread in patients with extensive involvement of the infratemporal fossa. All patients underwent surgery, and the most common approach was endoscope-assisted midface degloving. A total of 4 patients underwent craniotomy with an endoscope-assisted transfacial approach, which was single-stage surgery in 2 patients and a staged procedure in 2 patients. Blood transfusion was required in 53.6% of stage IIIb, 90% of stage IVa, and 100% of stage IVb patients. Residual tumor was present in 4 patients, and 3 patients developed recurrent disease. Postoperative radiotherapy was given to 5 patients. An algorithm for the surgical management of JNA with intracranial involvement was proposed on the basis of the authors' results.
In most cases, JNA with extradural intracranial extension can be completely excised with an endoscopic or endoscope-assisted transfacial approach, but a tumor with intracranial intradural extension requires tailored craniotomy along with a transfacial approach that can be done in single sitting or as a staged surgery. A small number of patients with gross cavernous extension receiving blood supply from a cavernous segment of the internal carotid artery are better suited for Gamma Knife or intensity-modulated radiation therapy of the residual lesion in the cavernous sinus.
本研究旨在探讨青少年鼻咽血管纤维瘤（JNA）患者颅内扩展的特征，并提出一种治疗方案。
对2013年1月至2023年1月期间所有接受手术的JNA患者进行回顾性病历审查，根据Fisch分期分类的Andrews修正版，将颅内扩展的病例分为IIIb期、IVa期和IVb期纳入研究。收集患者的就诊年龄、症状、影像学检查结果、颅内扩展途径、治疗方法及随访情况。
142例接受JNA手术的患者中，有40例（28.2%）出现颅内受累。所有患者均为男性，年龄在10至26岁之间，就诊时平均年龄为17岁。根据Andrews - Fisch分类，28例为IIIb期，10例为IVa期，2例为IVb期。颞下窝广泛受累的患者中，经眶上裂侵犯鞍旁是最常见的颅内扩散途径。所有患者均接受了手术，最常用的方法是内镜辅助中面部掀翻术。共有4例患者采用内镜辅助经面部入路开颅手术，其中2例为一期手术，2例为分期手术。IIIb期患者53.6%、IVa期患者90%、IVb期患者100%需要输血。4例患者有残留肿瘤，3例患者出现复发。5例患者接受了术后放疗。基于作者的研究结果，提出了一种JNA颅内受累的手术治疗方案。
在大多数情况下，硬膜外颅内扩展的JNA可通过内镜或内镜辅助经面部入路完全切除，但颅内硬膜内扩展的肿瘤需要定制开颅手术及经面部入路，可一期完成或分期进行。少数接受来自颈内动脉海绵窦段供血的巨大海绵窦扩展患者，更适合对海绵窦残留病变进行伽玛刀或调强放射治疗。
